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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_697_Библиотеки_им_академика_М_И_Перельмана.pdf
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- •Foreword I
- •Foreword II
- •Preface
- •Acknowledgments
- •Contents
- •1: History of the Treatment of Anorectal Malformations
- •1.1 Introduction
- •1.2 The Early Times
- •References
- •2: Basic Anatomy and Physiology of Bowel Control
- •2.1 Internal Sphincter
- •2.2 General Anatomic Principles in Anorectal Malformations
- •2.3 Nerves
- •2.4 Blood Supply
- •2.5 Basic Physiology Principles of Bowel Control
- •References
- •3: Prenatal Diagnosis
- •3.1 Male Fetuses
- •3.1.1 Abnormal Sacrum (Fig. 3.3)
- •3.1.2 Tethered Cord
- •3.1.3 Absent Kidney (Fig. 3.5)
- •3.1.4 Vertebral Anomalies
- •3.1.5 Hydronephrosis (Fig. 3.6)
- •3.2 Female Fetuses
- •3.2.2 Pelvic Cystic Mass
- •3.2.3 Cloacal Exstrophy
- •References
- •4: Neonatal Management
- •4.1 Introduction
- •4.2 Most Common Scenario
- •4.4 Physical Examination
- •4.4.1 Male Patients
- •4.5 Female Babies
- •4.6 Neonatal Management
- •4.7 Cloacal Exstrophy
- •References
- •5: Colostomy
- •5.1 Introduction
- •5.2 Stoma Locations
- •5.3 Ileostomies
- •5.4 To Divert or Not to Divert, That Is the Question
- •5.5 Recommended Types of Colostomies
- •5.5.1 Newborn Babies with Anorectal Malformations
- •5.6 Left Transverse Colostomy
- •5.7 Cecostomies
- •5.8 Creation of a Colostomy
- •5.8.1 Surgical Technique
- •5.9 Colostomy in Cases of Cloaca with Hydrocolpos
- •5.10 Other Types of Colostomies
- •5.11 Colostomy Care
- •5.12 Colostomy Closure
- •5.13 Surgical Technique
- •5.14 Errors and Complications in Colostomies
- •5.16 Prolapse
- •5.17 Surgical Treatment for Prolapse
- •5.18 Malposition of the Stomas
- •References
- •6: Imaging
- •6.1 Introduction
- •6.2 Prenatal Diagnosis
- •6.3 Neonatal Imaging
- •6.4.1 Anatomic Facts and Timing
- •6.5 The Old Invertogram
- •6.6 High-Pressure Distal Colostogram
- •6.7 Technique
- •6.8 Most Common Errors
- •6.9 Not Showing the Coccyx and the Sacrum During the Fluoroscopy Studies
- •6.11 Distal Colostogram in Cloacas
- •6.12 Monitoring Constipation
- •6.13 Radiology During the Bowel Management Program
- •6.14 Monitoring the Urinary Tract
- •References
- •7: Bowel Preparation in Pediatric Colorectal Surgery
- •7.1 Major Procedures
- •7.2 Primary Procedures for the Treatment of Anorectal Malformation During the Newborn Period
- •7.3 Primary Pull-Through in Newborn Patients with Hirschsprung’s Disease
- •7.4 Patients with Hirschsprung’s Disease with Enterocolitis After the Neonatal Period
- •7.5 Patients with Hirschsprung’s Disease Beyond the Neonatal Period, Without Enterocolitis
- •7.6 Colostomy Closures
- •References
- •8: Recto-perineal Fistula
- •8.2 Associated Defects
- •8.3 Diagnosis
- •8.3.1 Female Patients
- •8.3.2 Male Patients
- •8.4 Management
- •8.5 Dilatations
- •8.6 Cutback Operation
- •8.7 Minimal Posterior Sagittal Anoplasty
- •8.7.1 Male Patients
- •8.7.2 Surgical Technique
- •8.7.3 Female Patients
- •8.8 Postoperative Care
- •References
- •9: Rectourethral Bulbar Fistula
- •Introduction
- •Associated Defects
- •Posterior Sagittal Anorectoplasty
- •Surgical Technique
- •Functional Results
- •References
- •10: Rectourethral Prostatic Fistula
- •10.1 Introduction
- •10.2 Associated Defects
- •10.3 Surgical Repair
- •References
- •11: Recto-bladder Neck Fistula
- •11.2 Associated Defects
- •11.2.1 Sacral Defects
- •11.2.2 Spinal-Associated Defects
- •11.2.3 Urologic-Associated Defects
- •11.2.5 Neurosurgical-Associated Defects
- •11.2.6 Cardiovascular-Associated Defects
- •11.2.7 Other Associated Defects
- •11.3 Diagnosis
- •11.4 Treatment
- •11.4.1 Colostomy
- •11.4.2 Main Repair
- •11.4.3 Laparotomy
- •11.4.4 Laparoscopy
- •11.5 Special Problems
- •11.6 Functional Results
- •11.6.1 Fecal Control
- •11.6.2 Urinary Control
- •References
- •12: Imperforate Anus Without Fistula in Males and Females
- •12.1 Introduction
- •12.2 Anatomic Characteristics
- •12.3 Main Repair
- •12.4 Function and Results
- •References
- •13: Minimally Invasive Approach to Anorectal Malformations
- •13.1 Introduction
- •13.2 Males
- •13.3 Females
- •References
- •14: Rectal Atresia
- •14.1 Treatment
- •14.2 Surgical Repair
- •References
- •15: Rectovestibular Fistula
- •15.2 Associated Defects
- •15.2.1 Sacral
- •15.2.2 Spinal
- •15.2.3 Urologic
- •15.2.4 Gynecologic
- •15.2.5 Gastrointestinal
- •15.2.6 Tethered Cord
- •15.2.7 Cardiovascular
- •15.3 Diagnosis
- •15.4 Treatment
- •15.4.1 Colostomy or No Colostomy
- •15.5 Main Repair (Animation 15.1)
- •15.6 Complications
- •15.7 Functional Results
- •15.9 Surgical Technique
- •References
- •16: Cloaca, Posterior Cloaca and Absent Penis Spectrum
- •16.1 Cloaca
- •16.1.1.1 Associated Defects
- •16.1.1.2 Goals of Treatment
- •16.1.1.3 Neonatal Management
- •16.1.1.4 Main Repair
- •Cloacas with a Common Channel of Less Than 1 cm
- •Cloacas with a 1–3 cm Common Channel
- •Cloacas with a 3- to 5-cm Common Channel (Animation 16.3)
- •Carving of the Pubic Cartilage Maneuver
- •Separations of Vagina(s) from the Urinary Tract (Animation 16.3)
- •Vaginal Switch
- •Vaginal Replacement
- •Vaginal Replacement with Rectum
- •Vaginal Replacement with Colon
- •Vaginal Replacement with Small Bowel
- •Cloacas with Extremely Long Common Channels
- •16.1.1.5 Postoperative Care
- •16.1.2 Urologic Concerns
- •16.1.3 Gynecologic Concerns
- •16.1.4 Reoperations
- •16.1.4.1 Persistent Urogenital Sinus
- •16.1.4.3 Acquired Urethral Atresia or Stricture
- •16.1.4.4 Sequelae from Catastrophic Complications
- •16.1.5 Transpubic Approach
- •16.2 Posterior Cloaca and Absent Penis Spectrum
- •16.2.1 Surgical Repair
- •References
- •17: Cloacal Exstrophy and Covered Cloacal Exstrophy
- •17.1 Neonatal Approach
- •17.2 Pull-Through or “Permanent Stoma”
- •17.3 Covered Cloacal Exstrophy
- •References
- •18: General Principles for the Postoperative Management of Patients with Anorectal Malformations
- •18.1 General Care
- •18.2 Local Care
- •18.3 Anal Dilatations
- •18.4 Avoiding Constipation
- •18.5 Toilet Training
- •19: Postoperative Evaluation
- •References
- •20: Bowel Management for the Treatment of Fecal Incontinence
- •20.1 Introduction
- •20.2 Goals of the Bowel Management Program
- •20.3 Evaluation of the Patient for Bowel Management
- •20.5 Laxative Trial
- •20.6 About Our Program
- •20.7 Content of the Enema
- •20.8 Rationale to Change the Type of Enema
- •20.9 Bowel Management for the Treatment of Severe Diaper Rash
- •20.10 Bowel Management Through a Stoma
- •References
- •21: Operations for the Administration of Antegrade Enemas
- •21.1 Introduction
- •21.2 Our Preferred Technique
- •21.4 Continent Neo-appendicostomy
- •References
- •22: Reoperations
- •22.1 Introduction
- •22.4.1 Recurrent Fistula (17 Cases)
- •22.4.2 Persistent Rectourethral Fistula (24 Cases)
- •22.4.3 Acquired Fistula (9 Cases)
- •22.5 Posterior Urethral Diverticulum (32 Cases)
- •22.6 Acquired Rectal Atresia or Stenosis (83 Cases)
- •22.7 Presacral Masses
- •22.9 Prolapse
- •References
- •23: Urologic Problems in Anorectal Malformations
- •23.1 Introduction
- •23.2 Neonatal Approach
- •23.4 Most Common Urologic Abnormalities in Male Patients with Anorectal Malformations
- •23.4.1 Absent Kidney
- •23.4.2 Urethral Problems
- •23.6 Hypospadias
- •23.7 Ectopic Ureters in Males
- •23.8 Ectopic Ureters in Females
- •23.9 Ectopic Vas Deferens
- •23.10 Ectopic Verumontanum
- •23.11 Megalourethra
- •23.13 Neurogenic Bladder
- •23.14 Postoperative Problems
- •23.16 Sexual Problems
- •23.17 Tethered Cord
- •23.18 The Ultimate Concern, Kidney Function
- •References
- •24: Hirschsprung’s Disease
- •24.1 Introduction
- •24.2 Historical Review
- •24.3 Incidence, Inheritance, and Associated Anomalies
- •24.4 Pathogenesis
- •24.5 Genetics
- •24.6 Clinical Manifestations and Differential Diagnosis
- •24.7 Histologic Diagnosis
- •24.8 Differential Diagnosis
- •24.9 Early Management
- •24.10 Surgical Treatment
- •24.10.1 The Authors’ Approach
- •24.11 Total Colonic Aganglionosis
- •24.13 Problems, Complication, and Sequela Secondary to Operations for Hirschsprung’s Disease
- •24.13.1.1 Fecal Incontinence
- •24.13.2 Non-preventable Complications
- •24.13.3 Partially Preventable Complications
- •References
- •25: Idiopathic Constipation and Other Motility Disorders
- •25.2 Incidence, Social Impact, and Relevance
- •25.3 Etiology
- •25.3.2 Rectal Manometry
- •25.3.5 Botulinum Toxin Injection
- •25.4 Pathogenesis
- •25.5 Natural History and Clinical Manifestations
- •25.6 Diagnosis
- •25.6.1 Colonic Transit Time
- •25.6.2 The Evaluation of Severity: Search for Objective “Instruments”
- •25.7 Management
- •25.7.3 Electric Stimulation
- •25.8 Surgical Treatment
- •25.8.2 Colonic Resection
- •References
- •26: Posterior Sagittal Approach for the Treatment of Other Conditions
- •26.1 The Kraske Operation
- •26.2 Urogenital Sinus with Normal Rectum
- •26.3 Urogenital Sinus with Normal Rectum and Adrenal Hyperplasia
- •26.4 Acquired Urethral Atresia
- •26.5 Acquired Rectourethral Fistula
- •26.6 Giant Seminal Vesicle
- •26.7 Urethral Tumors
- •26.8 Acquired Rectovaginal Fistula
- •26.9 Rectal Tumors
- •26.10 Presacral Masses
- •26.11 Surgical Technique
- •26.12 Posterior Sagittal Approach, Its Application in Cases with Hirschsprung’s Disease
- •26.13 Vaginal Atresia with Normal Rectum
- •References
- •27: Miscellaneous Conditions
- •27.1 Part I: Perianal Abscess and Fistula

140
ab
Fig. 9.24 Dissection of the lateral walls of the rectum, removing all the white fascia, fat tissue, and extrinsic vessels.
( a ) Before removing the white fascia. ( b ) After removing the white fascia
9 Rectourethral Bulbar Fistula
The dissection continues, intermittently
switching from the lateral to the ventral dissection, until the rectum is completely separated
from the urinary tract (Fig. 9.25 ). At that point,
we estimate how much length is necessary in
order to bring the rectum down to suture it to the
skin with no tension. Rectourethral bulbar fi stula
cases require a minimal dissection on the rectum
to reach the skin, due to the fact that the rectum is
located rather low in the pelvis. This dissection is
performed in a circumferential manner.
Uniform traction is applied on all the multiple silk stitches, including the 5-0 silk stitches
that we placed originally when we opened the
posterior rectal wall and the multiple 6-0 silk
stitches. We like to say that “traction creates
a plane,” and by doing that, we can identify
bands that represent the vessels and nerves that
hold the rectum up in the pelvis (Animation
9.2 ). These tension bands are identifi ed as
grooves. The bands must be separated from the
rectal wall, burned, and divided. These vessels
must be burned carefully because they have a
tendency to bleed when they retract into the
pelvis (Fig. 9.26 ).
The perirectal dissection continues until we
feel that we have gained enough length of rectum
to bring it down to the perineum (Fig. 9.27 ). It is
rather unusual to have to open the peritoneum
when dealing with rectourethral bulbar fi stulas.
Opening the peritoneum is frequently necessary
in cases of rectoprostatic fi stulas but only rarely
in this defect.
Once we gain enough rectal length, we then
evaluate whether or not the patient needs tapering
of the rectum. This is a maneuver that we have
used very often in the past and we are using less
and less now. We believe that this is a manifestation of the fact that now patients are receiving
better colostomies (descending colon). An adequate colostomy decreases the frequency of
megarectum that we frequently saw in patients
that had transverse colostomies and were left
many months without a repair. Those patients
developed a megarectum that later translated into
severe constipation. We believe that patients are
now operated on earlier in life, and they have better colostomies (not transverse). Irrigating and
cleaning the distal bowel during the opening of
the colostomy result in a collapsed rectum (no

9.4 Surgical Technique
ab
141
Fig. 9.25 Submucosal dissection of the anterior rectal wall to protect the urinary tract. ( a ) Diagram. ( b ) Operative
megarectum); therefore, it is rather unusual now
to have to do tapering of these rectums. It is
almost impossible to irrigate and clean a distal
rectosigmoid through a transverse colostomy.
When a tapering is necessary, we should taper
the rectum to the size of the available space,
within the limits of the sphincter mechanism. The
posterior aspect of the rectum is resected accordingly (Fig. 9.28 ). One should not taper the ante-
rior rectal wall as this would leave a suture line
located against the urethral fi stula closure suture
line, which is a predisposing factor for a fi stula
formation. The tapering may include 30–60 % of
the rectal wall. The posterior rectal wall is
repaired with two layers of interrupted 5-0 Vicryl
sutures (Fig. 9.29 ).
The fi stula is closed with three or four interrupted stitches of 6-0 Vicryl sutures. Those
stitches take the white fascia that used to cover
the rectum and urethra. No special effort is made
to suture mucosa to mucosa at the fi stula orifi ce
(Fig. 9.30 ). In over thousand cases of male
patients with anorectal malformations operated
by us, we have never had a recurrence of a rectourethral fi stula.
The rectum is then placed within the limits of
the sphincter. The levator muscle is sutured
together behind the rectum in the midline with
interrupted 5-0 Vicryl sutures. This can be done
with the rectum located up and away from its
future location to facilitate the visualization of
the levator muscle (Fig. 9.31 ) or can be done with

142
ab
9 Rectourethral Bulbar Fistula
Fig. 9.26 Dividing and burning extrinsic vessels and bands of the rectum while applying traction, in order to pull it
down. ( a ) Diagram. ( b ) Operative
the rectum already in place, particularly if the
rectum is not very bulky. One can see the edge of
the levator muscle, or one can use the electrical
stimulator to be able to see it better.
We identify the junction between the levator
and the muscle complex at the place where they
create a 90° angle (Fig. 9.32 ). Actually, both
structures (levator and muscle complex) are part
of a continuum of muscle. This angle is less
noticeable in cases with a poor sphincter
mechanism.
Some patients with very poor sphincter mechanism (mainly rectoprostatic or recto-bladder
neck fi stula) simply have no available space
between the levator and the urethra. There is no
way to reconstruct the levator behind the rectum.
Also, in patients with a very fl at bottom, and
therefore very poor sphincter mechanisms, it is
very diffi cult to identify these sphincter structures. Fortunately, in cases of rectourethral bulbar
fi stula, this sphincter mechanism is easy to identify. The posterior edges of the muscle complex
from each side are sutured together in the midline
with interrupted 5-0 Vicryl sutures (Fig. 9.33 ).
These stitches take a bite of the posterior rectal
wall in order to anchor the rectum in a good position, and we think that that helps to prevent prolapse. The limits of the sphincters are electrically
determined and marked with temporary 5-0 silk
stitches (Fig. 9.34 ). The perineal body is recon-
structed, bringing together the anterior limits of
the muscle complex and the anterior limits of the
sphincter in those cases in which our posterior
sagittal incision was extended beyond the anterior limits of the sphincter (Fig. 9.35 ). The ischio-
rectal fossa is obliterated suturing the fat with

9.4 Surgical Technique
143
Fig. 9.27 Rectal dissection fi nished, the rectum reaches
the perineum comfortably, without tension
interrupted 5-0 Vicryl sutures. The same suture
material is used superfi cially to bring together the
parasagittal fi bers and subcutaneous tissue, putting a special emphasis in not taking the fi bers of
the parasagittal fi bers themselves with our
sutures, but rather bringing them together. U-type
of stitches placed parallel to the muscle fi bers
facilitates the maneuver (Fig. 9.36 ). The ano-
plasty is performed with 16 circumferential
stitches under slight tension.
The silk sutures used to pull the rectum are
separated into two, right and left sutures. An incision is made in the anterior rectal wall in the midline; a fi rst stitch of 6-0 Vicryl is placed, taking
skin, bowel, and skin again as a U-type of suture.
The same maneuver is done in the posterior corner of the anoplasty. The edges of the rectum are
resected (Fig. 9.36 ), and stitches are placed in a
radial (circumferential) fashion. The keys to
avoid dehiscence of the anoplasty are to avoid
excessive tension or devascularization and to be
sure that all the stitches are placed under the same
tension. Also, we want to be sure that the stitches
a b
Fig. 9.28 Rectal tapering in a case of megarectum. Approximately 40 % of the posterior aspect of the rectum is
resected. ( a ) Diagram. ( b ) Operative

144
ab
Fig. 9.29 The posterior rectal wall is repaired with two layers of interrupted sutures. ( a ) Before suturing. ( b ) After
suturing
9 Rectourethral Bulbar Fistula
Fig. 9.30 Fine absorbable sutures are used to close the
urethral side of the fi stula. Arrow shows the fi stula site
take full-thickness bowel, since sutures placed
taking only the mucosa do not hold well to the
skin. The sutures should be tied but not to the
point of cutting the tissue with excessive force.
Through the years, we have learned that the
blood supply of the rectum is well preserved provided the rectal wall integrity is respected and
remains intact. An intact rectal wall guarantees a
good intramural blood supply. Dissection of the
rectum should be performed, staying as close as
possible to the bowel wall, yet without injuring it.
That is particularly crucial in patients with rectoprostatic fi stulas or higher defects. In patients
with rectourethral bulbar fi stula, this usually does
not represent a problem because these patients
have a rectum that requires minimal circumferential dissection to reach the skin of the anal
dimple.
The skin incision is usually closed with subcuticular 5-0 Monocryl (Fig. 9.37 ). We use an anti-
biotic ointment for the anoplasty and the posterior
sagittal incision to be used for 5 days postoperatively. A Foley catheter is left in place for 1 week.
A “double diaper” technique is used for the Foley
catheter. The fi rst diaper has an orifi ce through
which the catheter is exteriorized; a second diaper
is used to receive the urine. In this way, we avoid
the use of a bulky Foley bag or “urinometer.” In
addition, the skin of the patient’s perineum is better protected. We have never seen a case of an
“ascending urinary tract infection” attributable to
this technique.
We try to leave the catheter in place for one
entire week. Sometimes, however, 3 or 5 days after

9.4 Surgical Technique
145
a
bc
Fig. 9.31 Sutures are lying down taking both edges of
the levator muscle. The rectum is pulled up in order to
have a more clear view of the levator muscle. ( a ) Diagram.
( b ) Operative. ( c ) The rectum was pulled down deeper
than the levator

146
9 Rectourethral Bulbar Fistula
Fig. 9.32 The limits between the levator muscle and the
muscle complex are arbitrarily determined at the place
where both structures form an angle. a angle at the junc-
tion of levator muscle complex, L levator, M muscle
complex
the operation, the parents describe that the babies
suffer from severe cramps and simultaneously the
parents can see urine coming out around the Foley
catheter. That is a characteristic manifestation of
bladder spasms. One can give them oxybutinin for
the treatment of these spasms, but it usually does
not work, and it is better to simply remove the
Foley catheter; by doing that, even after only 48 h
from the operation, we have never seen a problem.
The fact that the baby is voiding around the catheter means that the catheter is no longer needed.
Most patients, however, can tolerate the catheter
for one entire week. A week after surgery, the baby
comes to our clinic and we pull the catheter in the
morning to be sure that the patient can urinate well.
Not being able to urinate for a baby with a
good sacrum and a rectourethral bulbar fi stula
after the Foley is removed is a bad sign and usually is the result of a poor surgical technique. If
the baby was urinary continent, had a normal urethra, and cannot void after a posterior sagittal
operation, it means that there must have been an
intraoperative nerve injury. This is prevented by
following the basic principles of the operation,
mainly staying exactly in the midline during the
dissection of the rectum and being careful in the
a b
Fig. 9.33 The posterior edges of the muscle complex are sutured together, taking with the same suture a bite of the
posterior rectal wall. ( a ) Diagram. ( b ) Operative

9.4 Surgical Technique
a b
147
Fig. 9.34 The limits of the sphincter at the skin level
have been electrically determined and marked with
temporary silk stitches, prior to the levator reconstruction.
Fig. 9.35 The perineal body was reconstructed, bringing
together the anterior limits of the sphincter. This was done
prior to the pull-through of the rectum
( a ) Electrical stimulation to determine the limits. ( b ) The
limits of the sphincter marked with temporary silk stitches
Fig. 9.36 Anoplasty performed with 16 circumferential
stitches
area of dissection anteriorly between the rectum
and urinary tract. Also, a baby who had erections
prior to the operation and the parents describe
that they cannot see erections after the operation
can be interpreted in the same way.

148
9 Rectourethral Bulbar Fistula
Fig. 9.37 Subcutaneous tissue, dermis, and skin are
closed
Sometimes, those symptoms (urinary retention and absence of erections) are temporary and
patients recover, but it is still a very worrisome
sign. In patients with very abnormal sacra or
myelomeningocele, one can expect those kinds
of problems, but in patients with a normal sacrum,
they are considered iatrogenic and therefore
unacceptable.
Two weeks after surgery, the baby comes to the
clinic, and we show the parents how to perform
anal dilatations. We give them a copy of our protocol (See Chap. 5 ) that they are supposed to fol-
low religiously. Dilatation should not be painful;
they are uncomfortable but not painful if the operation was done correctly. Usually, about 2 months
after these operations, the colostomy is closed.
9.5 Functional Results
Ninety-seven patients were older than 3 years
and in contact with us, and 75 of them (78 %) had
voluntary bowel movements. Half of them occasionally had marks (soiling) in the underwear.
Ninety-two percent of patients are urinary continent. The soiling is usually a consequence of a
problem with constipation that has not been
treated well. The use of laxatives frequently takes
care of this problem.
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